Pediatric brainstem high grade glioma [Neurosurgery Wiki]

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=====Pediatric brainstem high grade glioma=====
Due to high-grade pediatric brainstem tumors’ relative rarity, most studies have been small clinical trials or single-institution studies
((Sun T, Wan W, Wu Z, Zhang J, Zhang L. Clinical outcomes and natural history of pediatric brainstem tumors: With 33 cases follow-ups. Neurosurg Rev. 2013;36:311–9.))
((Lewis J, Lucraft H, Gholkar A. UKCCSG study of accelerated radiotherapy for pediatric brain stem gliomas. United Kingdom Childhood Cancer Study Group. Int J Radiat Oncol Biol Phys. 1997;38:925–9.))
((Ueoka DI, Nogueira J, Campos JC, Maranhão Filho P, Ferman S, Lima MA. Brainstem gliomas – retrospective analysis of 86 patients. J Neurol Sci. 2009;281:20–3.))
((Wolff JE, Rytting ME, Vats TS, Zage PE, Ater JL, Woo S, et al. Treatment of recurrent diffuse intrinsic pontine glioma: The MD Anderson Cancer Center experience. J Neurooncol. 2012;106:391–7.))
((Garzón M, García-Fructuoso G, Guillén A, Suñol M, Mora J, Cruz O. Brain stem tumors in children and adolescents: Single institutional experience. Childs Nerv Syst. 2013;29:1321–31.))
===2016===
Between January 2000 and December 2014, 29 patients were identified. The mean age at diagnosis was 8.4 years (range 2 months to 25 years), and 17 (59%) patients were male. The most common presenting signs and symptoms were cranial neuropathies (n = 24; 83%), hemiparesis (n = 12; 41%), and ataxia or gait disturbance (n = 10; 34%). There were 18 glial and 11 embryonal tumors. Of the glial tumors, 5 were radiation-induced and 1 was a malignant transformation of a previously known low-grade tumor. Surgical intervention consisted of biopsy alone in 12 patients and some degree of resection in another 15 patients. Two tumors were diagnosed postmortem. The median overall survival for all patients was 196 days (range 15 to 3999 days). There are currently 5 (17%) patients who are still alive: 1 with an anaplastic astrocytoma and the remaining with embryonal tumors. 

In general, malignant non-DIPG tumors of the brainstem carry a poor prognosis. However, maximal cytoreductive surgery may be an option for select patients with focal tumors. Long-term survival is possible in patients with nonmetastatic embryonal tumors after multimodal treatment, most importantly maximal resection
((Klimo P Jr, Nesvick CL, Broniscer A, Orr BA, Choudhri AF. Malignant brainstem 
tumors in children, excluding diffuse intrinsic pontine gliomas. J Neurosurg
Pediatr. 2016 Jan;17(1):57-65. doi: 10.3171/2015.6.PEDS15166. Epub 2015 Oct 16.
PubMed PMID: 26474099.
)).

===2015===
In a cohort of 124 patients, those with AA had a median survival of 13 months and those with GBM 9 months. Higher-grade tumors were associated with statistically significantly increased mortality (hazard ratio [HR]: 1.74, confidence intervals [CIs]: 1.17-2.60). Surgical intervention was associated with a significantly lower mortality, either alone (HR: 0.14, CI: 0.04-0.5) or in combination with radiation (HR: 0.35, CI: 0.15-0.82). Radiation therapy alone was significantly associated with decreased mortality within the first 9 months after diagnosis but not with overall mortality. No demographic characteristics were significantly associated with mortality.

Outcome remains poor in the pediatric high-grade brainstem glioma population. Survival is correlated with lower-grade tumor histology, radiation therapy only in the first 9 months after diagnosis, and surgical resection
((Lam S, Lin Y, Auffinger B, Melkonian S. Analysis of survival in pediatric
high-grade brainstem gliomas: A population-based study. J Pediatr Neurosci. 2015 
Jul-Sep;10(3):199-206. doi: 10.4103/1817-1745.165656. PubMed PMID: 26557158;
PubMed Central PMCID: PMC4611886.)).